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Tidsskrift For Den Norske Laegeforening... Apr 2020Although very rare, cardiac sarcoma is the most common malignant cardiac tumour. Cardiac tumours may present with constitutional symptoms and this can lead to delayed...
BACKGROUND
Although very rare, cardiac sarcoma is the most common malignant cardiac tumour. Cardiac tumours may present with constitutional symptoms and this can lead to delayed diagnosis.
CASE PRESENTATION
A woman in her late twenties was admitted to the acute psychiatric ward with suspected depressive psychosis. Although she presented with insomnia, auditory hallucinosis and paranoid delusions, her main symptoms were dyspnoea, tachycardia and exhaustion. She was discharged after 17 days and admitted directly to the medical ward for further examination. A thorough somatic examination, including diagnostic imaging, revealed a tumour in the left atrium obstructing the mitral ostium in the diastole, resulting in orthopnoea. She had urgent cardiac surgery and the histology was consistent with an intimal sarcoma.
INTERPRETATION
This case illustrates a rare disease presenting with atypical symptoms. Her psychiatric symptoms resolved after surgery and oncologic treatment of her cardiac tumour.
Topics: Female; Heart Neoplasms; Hospitalization; Humans; Sarcoma
PubMed: 32321220
DOI: 10.4045/tidsskr.19.0455 -
BMJ Case Reports Oct 2018Pericardial sarcomas are extremely rare aggressive neoplasms. Non-specific symptoms and incidental discovery are usually the rule. Multimodality imaging is extremely...
Pericardial sarcomas are extremely rare aggressive neoplasms. Non-specific symptoms and incidental discovery are usually the rule. Multimodality imaging is extremely important for diagnosis and tissue characterisation of all cardiac masses. Despite treatment, pericardial sarcomas are considered extremely fatal. We encountered a 27-year-old female patient who presented to our facility with progressive dyspnoea. On examination, clinical signs of cardiac tamponade were appreciated, transthoracic echocardiography revealed a tamponading pericardial effusion and a large heterogeneous pericardial mass. Pericardiocentesis revealed haemorrhagic fluid. Subsequently, CT revealed a pericardial mass compressing the right atrium. Excision biopsy showed a well-circumscribed mass, and cut sections showed friable grey-white tissue with areas of haemorrhage and necrosis. Pathological examination confirmed the diagnosis of high-grade undifferentiated sarcoma of the pericardium. The patient was started on adjuvant chemotherapy and radiotherapy. Follow-up after 1 year showed no relapse.
Topics: Adult; Cardiac Tamponade; Chemotherapy, Adjuvant; Combined Modality Therapy; Diagnosis, Differential; Dyspnea; Female; Humans; Pericardium; Radiotherapy; Sarcoma; Tomography, X-Ray Computed
PubMed: 30297493
DOI: 10.1136/bcr-2018-226112 -
The Annals of Thoracic Surgery Jul 2010The presentation, management, and outcome of patients with primary cardiac sarcomas are not well defined. Furthermore, the role of adjuvant therapy has not been...
BACKGROUND
The presentation, management, and outcome of patients with primary cardiac sarcomas are not well defined. Furthermore, the role of adjuvant therapy has not been delineated in the management of primary cardiac sarcomas.
METHODS
Patients with primary cardiac sarcoma and noncardiac sarcoma, diagnosed between 1988 and 2005, were identified in the Surveillance, Epidemiology, and End Results (SEER) database. Clinical characteristics and outcomes of primary cardiac sarcoma were defined and compared with the characteristics of noncardiac sarcomas. Univariate and multivariate methods were used to identify factors associated with primary cardiac sarcoma survival.
RESULTS
Compared with noncardiac sarcomas, primary cardiac sarcomas were found to occur in a younger age group and were more likely to present with advanced disease. Primary cardiac sarcomas were ten times more likely to be vessel-derived (eg, angiosarcoma), comprising almost half of all cases. Median overall survival for cardiac sarcoma patients was 6 months whereas that for noncardiac sarcoma patients was significantly longer at 93 months (p < 0.001). Furthermore, cardiac sarcoma patients who underwent surgery had a median survival of 12 months whereas those who did not undergo surgery had a median survival of 1 month (p < 0.001).
CONCLUSIONS
Cardiac sarcomas are a distinct, rare subset of soft tissue sarcomas with a poor prognosis. Surgery continues to be the central component of successful management. Future clinical efforts should be directed at developing approaches to permit safe radical excision and, potentially, developing effective adjuvant therapy.
Topics: Adult; Aged; Female; Heart Neoplasms; Humans; Male; Middle Aged; SEER Program; Sarcoma; Survival Analysis; United States; Young Adult
PubMed: 20609770
DOI: 10.1016/j.athoracsur.2010.03.065 -
The Journal of Thoracic and... Jul 2021
Topics: Heart Neoplasms; Humans; Sarcoma; Thymus Neoplasms
PubMed: 32093871
DOI: 10.1016/j.jtcvs.2020.01.033 -
Methodist DeBakey Cardiovascular Journal 2010Primary tumors of the heart are uncommon, with roughly 75% benign and 25% malignant. Most of the malignant tumors are sarcomas and historically have had a very poor...
Primary tumors of the heart are uncommon, with roughly 75% benign and 25% malignant. Most of the malignant tumors are sarcomas and historically have had a very poor prognosis. These tumors tend to occur in young patients with a mean age of 40 years. Making a diagnosis of cardiac sarcoma can be difficult due to its rarity and the nature of the symptoms. For left heart sarcomas, almost all patients are symptomatic by the time the diagnosis has been made. Symptoms are dependent on the location and the extent of the tumor and are not related to tumor histology; similarly, histologic cell type has not been found to be related to prognosis in most studies. We therefore previously proposed a classification system for primary cardiac sarcoma based on anatomic location, dividing primary cardiac sarcoma into right heart, pulmonary artery and left heart sarcomas. Left heart sarcoma presents a technical anatomic challenge: the left atrium, being the posterior heart chamber, allows somewhat limited access using routine surgical approaches. The role of chemotherapy or radiotherapy remains unclear and unproven, leaving complete surgical resection as the only mode of therapy with a proven survival benefit. Our review of the published literature showed frequent local recurrence and poor long-term survival in left heart sarcomas. Our hypothesis was that the left atrium had limited anatomic accessibility for large complex resections and reconstructions, and this led cardiac surgeons to do a more limited tumor removal with an increased chance of local recurrence and a detrimental effect on survival. To address this technical challenge, our group introduced the surgical technique of cardiac explantation, ex vivo tumor resection, cardiac reconstruction, and subsequent cardiac reimplantation or cardiac autotransplantation for left heart sarcoma in an attempt to improve the completeness of local resection, decrease local recurrence, and extend patient survival. This review discusses the approach of the cardiac sarcoma group at the Methodist DeBakey Heart & Vascular Center and the MD Anderson Cancer Center to the diagnosis and treatment of left heart sarcoma, as well as our current patient outcomes.
Topics: Cardiac Surgical Procedures; Heart Neoplasms; Humans; Replantation; Sarcoma; Time Factors; Treatment Outcome
PubMed: 20834212
DOI: 10.14797/mdcj-6-3-49 -
The Journal of Thoracic and... Apr 2017
Topics: Heart Atria; Humans; Sarcoma; Soft Tissue Neoplasms
PubMed: 28131512
DOI: 10.1016/j.jtcvs.2016.12.018 -
Methodist DeBakey Cardiovascular Journal 2010
Topics: Cardiac Surgical Procedures; Chemotherapy, Adjuvant; Heart Neoplasms; Humans; Sarcoma; Time Factors; Treatment Outcome
PubMed: 20834209
DOI: 10.14797/mdcj-6-3-35 -
International Heart Journal Mar 2021In this study, we present the case of a 34-year-old man who was diagnosed with primary cardiac angiosarcoma 1 month after hospital admission. Cardiac angiosarcoma is a...
In this study, we present the case of a 34-year-old man who was diagnosed with primary cardiac angiosarcoma 1 month after hospital admission. Cardiac angiosarcoma is a relatively rare disease that can be easily misdiagnosed as pneumonia or other diseases. Although surgery is the preferred treatment to prolong survival time, highly malignant tumors with local infiltration and systemic metastasis can lead to poor prognosis.
Topics: Adult; Diagnosis, Differential; Echocardiography; Heart Atria; Heart Neoplasms; Hemangiosarcoma; Humans; Male; Tomography, X-Ray Computed
PubMed: 33731530
DOI: 10.1536/ihj.20-581 -
Anatolian Journal of Cardiology Feb 2021Primary cardiac sarcoma, a rare tumor with an aggressive course and imprecise prognosis, constitutes over 95% of all malignant cardiac tumors. Given the sparsely...
OBJECTIVE
Primary cardiac sarcoma, a rare tumor with an aggressive course and imprecise prognosis, constitutes over 95% of all malignant cardiac tumors. Given the sparsely available evidence, there is a paucity of information regarding current knowledge on cardiac sarcoma. This study aimed to determine the incidence and incidence-based rates, patient characteristics, treatment modalities, and survival factors of cardiac sarcoma.
METHODS
A retrospective analysis of the incidence, incidence-based mortality rates and characteristics of cardiac sarcoma between 1975 and 2016 was carried out using the Surveillance, Epidemiology, and End Results (SEER) database. The National Cancer Institute's Joinpoint Regression program was used to calculate the Annual Percentage Changes (APC). Univariate and multivariate regression analysis were used to determine the survival characteristics.
RESULTS
A total 408 patients were identified for the incidence analysis, while 385 eligible patients were identified for the survival analysis. The mean age at diagnosis was 46.3±17.9 years. The incidence rate (per 100.000 per year) of cardiac sarcoma within the indicated years was 0.22, with an increased APC of 1.7 (p=0.013, 95% CI=0.5-2.9). A total of 251 (61.5%) patients underwent surgery, 93 (22.8%) patients received adjuvant radiotherapy, and 197 (50.2%) patients received chemotherapy. Surgical resection, chemotherapy, stage of tumor, and younger age significantly improved the survival outcomes (p<0.001).
CONCLUSION
Cardiac sarcoma is a rare type of soft tissue sarcomas with poor prognosis. Over the past 30 years, the incidence of cardiac sar-coma has been on the increase. Surgery remains the mainstay of management. Further studies are needed to compare different diagnostic and treatment modalities so as to ascertain the best treatment option that would enhance survival and prognosis of cardiac sarcoma.
Topics: Heart Neoplasms; Humans; Incidence; Prognosis; Retrospective Studies; SEER Program; Sarcoma
PubMed: 33583817
DOI: 10.14744/AnatolJCardiol.2020.78107 -
Archives of Pathology & Laboratory... Apr 2012Primary cardiac synovial sarcoma is an uncommon malignant neoplasm, with only a handful of cases reported in the English literature to date. Synovial sarcomas have also... (Review)
Review
Primary cardiac synovial sarcoma is an uncommon malignant neoplasm, with only a handful of cases reported in the English literature to date. Synovial sarcomas have also been described at other unusual sites, such as the heart, pleuropulmonary region, kidney, prostate, liver, mediastinum, retroperitoneum, gastrointestinal tract, and peripheral nerve. For synovial sarcomas that arise at these unusual locations, definitive diagnosis is challenging and requires use of ancillary diagnostic procedures, such as immunohistochemistry, electron microscopy, and molecular genetic techniques, for confirmation of diagnosis. The nonrandom occurrence of t(X;18) has been found consistently in synovial sarcomas. It has also been found as a sole cytogenetic abnormality in some cases, suggesting it as a key molecular event in tumor development. This review highlights salient features of primary cardiac synovial sarcoma and the associated diagnostic challenges.
Topics: Chromosomes, Human, Pair 18; Chromosomes, Human, X; Diagnosis, Differential; Heart Neoplasms; Humans; Immunohistochemistry; Microscopy, Electron; Molecular Biology; Prognosis; Sarcoma, Synovial; Translocation, Genetic
PubMed: 22458908
DOI: 10.5858/arpa.2011-0008-RS